AB0651 Clinical Impact and Prognosis of cryoglobulinemia and cryofibrinogenemia in Systemic Sclerosis
Authors: S. De Almeida Chaves, P. Benedicte, T. Porel, E. Bories, D. Adoue, L. Astudillo, L. Alric, A. Huart, M. Michaud, D. Ribes, G. Prevot, L. Sailler, F. Gaches, G. Pugnet
Journal: Annals of the Rheumatic Diseases (2022)

Background Systemic sclerosis (SSc) is reported to be a secondary cause of cryoglobulinemia as well as cryofibrinogenemia. However, prevalence, clinical implication and associated pronostic of cryoprecipitates in SSc are unknown. Objectives To describe the prevalence, the phenotype and evaluate the prognosis of cryoglobulinemia and/or cryofibrinogenemia associated with systemic sclerosis. Methods We included all adult (≥18 years) consecutive SSc patients from the Systemic Scleroderma Toulouse Cohort (SSTC) [1] for whom a cryoglobulin and/or cryofibrinogen measurement was carried out at the immunology laboratory of the Toulouse University Hospital between June 1, 2005 and May 31, 2018 and at least one follow-up visit. We compared SSc-patients characteristics’ with and without cryoglobulinemia &gt; 50 mg/l and with and without cryofibrinogenemia. Survival analysis based on presence of cryoglobulin cryofibrinogen was performed using the Kaplan-Meier method. Univariable and multivariable Cox proportional hazards models (ascending step-by-step method) were used to determine baseline variables associated with cryoglobulin or cryofibrinogen presence. Results 166 patients were included in the study. 74.6% of patients were women, with a average age at diagnosis of 51.2 years-old. 24% were diffuse cutaneous subtypes and 71.6% limited cutaneous subtypes. Anti-centromere and anti-Scl70 were respectively positive in 44.5% and 21.6% of cases. All these patients were assessed for cryoglobulin detection and 75 cryofibrinogen detection in serum. 43.3% had a cryoglobulinemia &gt;50 mg/l. 46.6% had cryofibrinogenemia. Patients with cryoglobulinemia &gt;50 mg had more cardiac diastolic involvement (22.8% vs. 5.1% p=0.0395). In the multivariate analysis, diastolic involvement (HR=6.23; p=0.0331) was an independent predictor of cryoglobulin &gt;50 mg/l. Survival at 10 years was better for patients with cryoglobulinemia &gt;50 mg/l (log-rank 0.0363) (Figure 1). Survival at 5 and 10 years was 97.6% and 88.8% respectively in patients with cryoglobulinemia &gt;50 mg/l versus 91.9% and 78.4% in patients with cryoglobulinemia &lt;50 mg/l. In cox regression model adjusted for gender, age and type of systemic sclerosis, cryoglobulin &gt;50 mg/l was negatively associated with mortality (HR: 0.09; p=0.03). The presence of cryofibrinogenemia was not associated with any clinical, biological or morphological features. In the multivariate analysis, no variable was predictive of the presence of cryofibrinogenemia in patients with SSc. The presence of cryofibrinogenemia had no influence on the mortality of these patients. Conclusion In SSc patients, the presence of cryoglobulin &gt;50 mg/l is an independent predictive factor of cardiac diastolic involvement and is associated with a better survival. However, cryofibrinogenemia does not influence clinical phenotype or impact mortality in SSc patients. References [1]De Almeida Chaves S, Porel T, Mounié M, Alric L, Astudillo L et al. Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality. Arthritis Res Ther. 2021 Dec 7;23(1):295. doi: 10.1186/s13075-021-02672-y. Disclosure of Interests None declared